Long-term experience of bosentan for treating ulcers and healed ulcers in systemic sclerosis patients

Peña-Lefebvre, P. García de la; Rubio, Silvia; Expósito, M Valero; Carmona, Loreto; Gámir, M.L. Gámir; Gutiérrez, J. Beltrán; Díaz-Miguel, C.; Martinez, Juan; Mendoza, Alejandro

doi:10.1093/rheumatology/ken001

Cited by 68 publications

(35 citation statements)

References 8 publications

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“…Other small series of patients showed the beneficial effects of bosentan on treating DU in SSc 7 . A recent open-label study of 15 patients with SSc and DU using bosentan for a median period of 24 months revealed that bosentan was safe and effective in these patients 10 . Ours is the longest prospective study that has evaluated the number of healed DU and new ulcer formation.…”

Section: Rheumatologymentioning

confidence: 99%

Bosentan for Digital Ulcers in Patients with Systemic Sclerosis: A Prospective 3-year Followup Study

Tsifetaki¹,

Botzoris²,

Alamanos³

et al. 2009

J Rheumatol

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Section: Rheumatologymentioning

confidence: 99%

Bosentan for Digital Ulcers in Patients with Systemic Sclerosis: A Prospective 3-year Followup Study

Tsifetaki¹,

Botzoris²,

Alamanos³

et al. 2009

J Rheumatol

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“…And because there are no vasodilator drugs that have specific actions only on the pulmonary circulation, it raises the possibility that their effects on exercise performance could be mediated by changes elsewhere in the circulatory system, such as the peripheral vasculature. Studies have shown salutary effects of the endothelin receptor antagonists on the systemic circulation in patients with hypertension, 55 Raynaud phenomena 56 and fingertip ulceration, 57 and with prostacyclins in chronic limb ischemia 58 (Table 2). …”

Section: Prostacyclinsmentioning

confidence: 99%

The Effects of Vasodilators in Pulmonary Hypertension

Rich

2009

Circ: Heart Failure

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W hen primary pulmonary hypertension (PPH) was first described in the medical literature, it was characterized from a cardiac catheterization on a young woman who had an elevated pulmonary arterial pressure of unknown origin which, after the intravenous administration of acetylcholine, promptly fell. 1 The sentinel description of this phenomenon led to the acceptance of PPH as a medical entity in which inappropriate pulmonary vasoconstriction was a central feature. 2 Since then many series of patients with PPH (now referred to as idiopathic pulmonary arterial hypertension [IPAH]) have been published which document a variable ability to respond to acute vasodilator challenge. Although, the approach to therapy has emphasized the initial classification of responder or nonresponder, most patients demonstrate only a small decrease in pulmonary pressure (PAP) in response to vasodilators. 3 However, vasodilators are widely prescribed in patients who are nonresponders, based on clinical trials that established the response to a 6-minute walk (6 MW) test as the primary end point. 4 The consistent improvement in 6 MW reflects the complexity of exercise intolerance, which may be strongly influenced not only by cardiopulmonary factors, but by peripheral factors such as the muscle ergoreflex. 5 Who is a Responder?In the current era, patients with PAH 6 who undergo acute vasoreactivity testing at the time of diagnosis are classified as responders or nonresponders based on the change in PAP from select agents. 7 The definition of a responder has changed many times, the most recent being a fall in the mean PAP of at least 10 mm Hg to a level below 40 mm Hg without any fall in cardiac output. 4 However, the response is in reality a continuum, and not an all or none phenomenon. It is notable that in 2 large series of patients who were deemed acute responders, the characteristic of those who had near perfect survival on calcium blockers (CCB) for up to 18 years had an acute fall in mean PAP of 39% and an acute fall in pulmonary vascular resistance (PVR) of 50%. 3,8 It is remarkable how similar the experiences from the 2 series are, supporting the validity of the observations. Who is a Nonresponder?The majority of patients with IPAH are nonresponders. Although this implies lack of vasoreactivity, this is clearly not the case. In an observational study of patients with IPAH who were monitored with a pulmonary artery catheter over 6 hours without an intervention, considerable variability was noted in the PAP (Ϯ8%) and PVR (Ϯ13%), suggesting that changing vasomotor tone is common in these patients. 9 In the 2 series on long-term calcium blocker therapy, the nonresponders also had an acute fall in mean PAP and PVR, but of a lesser magnitude than the responder group. In one series, the fall in PAP was 8%, and fall in PVR was 17%. 8 In the other, nonresponders were noted to have a fall in PVR of less than 20%. 3 Thus, the characterization of a patient as a nonresponder actually means a minimal response, not lack of response. Although the...

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“…Although no healing of preexistent ulcers has been observed in the RAPIDS-1 study, several authors reported on cases with a marked improvement of digital ulcers [3,4,5]. In a long-term prospective noncontrolled study, bosentan showed a significant decrease in the number of ulcers [6]. …”

Section: Discussionmentioning

confidence: 99%

Successful Treatment of Systemic-Sclerosis-Related Digital Ulcers with a Selective Endothelin Type A Receptor Antagonist (Sitaxentan)

Gholam

Sehr²,

Enk³

et al. 2009

Dermatology

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Digital ulcers represent a major vascular complication of systemic sclerosis (SSc) and can be difficult to treat with the common vasodilators. Herein, we report on a 39-year-old patient with recalcitrant SSc-related digital ulcers treated successfully with sitaxentan (100 mg q.d.), a selective endothelin type A receptor antagonist. During the 6 months of treatment, we noticed a remarkable decrease in pain, a nearly complete healing of the preexistent ulcers and no development of new ulcers. No side effects were noted.

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Long-term experience of bosentan for treating ulcers and healed ulcers in systemic sclerosis patients

Abstract: Bosentan may be a safe long-term alternative for treating the recurrence of skin ulcers and healed ulcers in SSc patients.

Cited by 68 publications

References 8 publications

Bosentan for Digital Ulcers in Patients with Systemic Sclerosis: A Prospective 3-year Followup Study

Bosentan for Digital Ulcers in Patients with Systemic Sclerosis: A Prospective 3-year Followup Study

The Effects of Vasodilators in Pulmonary Hypertension

Successful Treatment of Systemic-Sclerosis-Related Digital Ulcers with a Selective Endothelin Type A Receptor Antagonist (Sitaxentan)

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